OBJECTIVE: To emphasize the importance of chest X-Rays in the diagnosis of Scimitar Syndrome. This anomaly is usually characterized by the presence of anomalous venous drainage of the right lung to the inferior vena cava, abnormal bronchial segmentation, and alterations of pulmonary artery and right lung vascularization.METHODS: The authors report a case of Scimitar Syndrome in an 8 year-old-child, discussing its diagnostic methods and surgical treatment.RESULTS: The child presented good outcome after surgical therapy, with an uneventful follow-up.CONCLUSION: The Scimitar Syndrome is a rare anomaly, which may present few clinical manifestations. The chest X-Ray is a very relevant tool in the screening of this disease, since it discloses a persisting right paracardiac image, as a result of abnormal right pulmonary venous drainage to inferior vena cava ("Scimitar sign").